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HERO ID
6858833
Reference Type
Journal Article
Subtype
Review
Title
Chronic Thromboembolic Pulmonary Hypertension: Evolving Therapeutic Approaches for Operable and Inoperable Disease
Author(s)
Mahmud, E; Madani, MM; Kim, NH; Poch, D; Ang, L; Behnamfar, O; Patel, MP; Auger, WR
Year
2018
Is Peer Reviewed?
Yes
Journal
Journal of the American College of Cardiology
ISSN:
0735-1097
EISSN:
1558-3597
Publisher
ELSEVIER SCIENCE INC
Location
NEW YORK
Volume
71
Issue
21
Page Numbers
2468-2486
Language
English
PMID
29793636
DOI
10.1016/j.jacc.2018.04.009
Web of Science Id
WOS:000432962100012
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH), a rare consequence of an acute pulmonary embolism, is a disease that is underdiagnosed, and surgical pulmonary thromboendarterectomy (PTE) remains the preferred therapy. However, determination of operability is multifactorial and can be challenging. There is growing excitement for the percutaneous treatment of inoperable CTEPH with data from multiple centers around the world showing the clinical feasibility of balloon pulmonary angioplasty. Riociguat remains the only approved medical therapy for CTEPH patients deemed inoperable or with persistent pulmonary hypertension after PTE. We recommend that expert multidisciplinary CTEPH teams be developed at individual institutions. Additionally, optimal and standardized techniques for balloon pulmonary angioplasty need to be developed along with dedicated interventional equipment and appropriate training standards. In the meantime, the percutaneous revascularization option is appropriate for patients deemed inoperable in combination with targeted medical therapy, or those who have failed to benefit from surgery.
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